S-100 Immuno-Positive Low Grade Myofibroblastic Sarcoma of Nasal Cavity: A Rare Case Presentation and Review of Literature.

Introduction: Myofibroblastic neoplasms comprise a spectrum of benign/malignant neoplasms. Only low-grade malignant forms have been reproducibly characterized as a diagnostic entity in the WHO classification. Low grade myofibroblastic sarcoma (LGMFS) confined to the nasal cavity is extremely rare. Objective: To review previously reported cases of nasal cavity LGMFS and provide a better insight regarding its clinical and immunohistochemical features. Data synthesis: A review was performed involving two databases (PubMed and Google Scholar). Four cases of nasal cavity LGMFS were included. The lesion showed no gender or nasal-side predilection. All cases underwent wide excision. None showed distant metastasis while half recurred locally. Histologically, mitotic rate ranged from 1 to 3/10 high-power-field (HPF) and none exhibited spontaneous necrosis. Immuno-expression of calponin, smooth muscle actin (SMA) and vimentin were seen in either all four or three-fourth of cases. Diffuse S-100 expression was a unique finding in present case and not reported previously, that caused a diagnostic dilemma with schwannomas. Conclusion: LGMFS of nasal cavity is extremely rare. A wide resection is the primary treatment of choice. Adjuvant therapies (chemotherapy or radiotherapy) are of uncertain significance. Distant metastasis is rather unusual. Calponin, SMA and vimentin are highly sensitive immuno-markers. Diffuse S-100 expression is a possible finding. Mitotic rate < 6/10 HPF and absence of spontaneous necrosis are characteristic indolent features differentiating from high grade lesions. Trifecta of clinical and morphological features plus immunohistological phenotype, are sufficient for a definitive diagnosis. Electron microscopy is the most definitive confirmation test, however, should be reserved only for equivocal/atypical immunostaining pattern.

Factors Associated with Mortality in Coronavirus-Associated Mucormycosis: Results from Mycotic Infections in COVID-19 (MUNCO) Online Registry.

BACKGROUND: COVID-19-associated mucormycosis (CAM) is associated with high morbidity and mortality. MUNCO is an international database used to collect clinical data on cases of CAM in real time. Preliminary data from the Mycotic Infections in COVID-19 (MUNCO) online registry yielded 728 cases from May to September 2021 in four South Asian countries and the United States. A majority of the cases (694; 97.6%) consisted of a mucormycosis infection. The dataset allowed for the analysis of the risk factors for adverse outcomes from CAM and this analysis is presented in this paper. METHODS: The submission of cases was aided by a direct solicitation and social media online. The primary endpoints were full recovery or death measured on day 42 of the diagnosis. All patients had histopathologically confirmed CAM. The groups were compared to determine the contribution of each patient characteristic to the outcome. Multivariable logistic regression models were used to model the probability of death after a CAM diagnosis. RESULTS: The registry captured 694 cases of CAM. Within this, 341 could be analyzed as the study excluded patients with an unknown CAM recovery status due to either an interruption or a lack of follow up. The 341 viable cases consisted of 258 patients who survived after the completion of treatment and 83 patients who died during the period of observation. In a multivariable logistic regression model, the factors associated with an increased risk of mortality include old age (OR = 1.04, 95% CI 1.02-1.07, p = 0.001), history of diabetes mellitus (OR 3.5, 95% CI 1.01-11.9, p = 0.02) and a lower BMI (OR 0.9, 95% CI 0.82-0.98, p = 0.03). Mucor localized to sinus disease was associated with 77% reduced odds of death (OR = 0.23, 95% CI 0.09-0.57, p = 0.001), while cerebral mucor was associated with an increased odds of death (OR = 10.96, 95% CI 4.93-24.36, p = ≤0.0001). CONCLUSION: In patients with CAM, older age, a history of diabetes and a lower body mass index is associated with increased mortality. Disease limited to the sinuses without a cerebral extension is associated with a lower risk of mortality. Interestingly, the use of zinc and azithromycin were not associated with increased mortality in our study.

Emergence of Invasive Fungal Rhinosinusitis in Recently Recovered COVID-19 Patients.

OBJECTIVE: The risk of invasive fungal rhinosinusitis is increased in Coronavirus disease 2019 (COVID-19) because of its direct impact in altering innate immunity and is further exacerbated by widespread use of steroids/antibiotics/monoclonal antibodies. The study aims to describe this recently increased clinical entity in association with COVID-19. METHOD: A prospective, longitudinal study including patients diagnosed with acute invasive fungal rhinosinusitis (AIFRS) who recently recovered from COVID-19 infection or after an asymptomatic carrier state. A single-center, descriptive study investigating demographic details, clinical presentation, radio-pathological aspects, and advocated management. RESULT: A total of 21 patients were included with a mean age of 49.62 years (SD: 14.24). Diabetes mellitus (DM) was the most common underlying disorder (90.48%), and 63.16% of all patients with DM had a recent onset DM, either diagnosed during or after COVID-19 infection. Nineteen patients (90.48%) had recently recovered from active COVID-19 infection, and all had a history of prior steroid treatment (oral/parenteral). Remaining 2 patients were asymptomatic COVID-19 carriers. Surprisingly, 2 patients had no underlying disorder, and 5 (23.81%) recently received the Covishield vaccine. Fungal analysis exhibited Mucor (95.24%) and Aspergillus species (14.29%). Most common sign/symptom was headache and facial/periorbital pain (85.71%), followed by facial/periorbital swelling (61.90%). Disease involvement: sinonasal (100%), orbital (47.62%), pterygopalatine fossa (28.58%), infratemporal fossa (14.29%), intracranial (23.81%), and skin (9.52%). Exclusive endoscopic debridement and combined approach were utilized in 61.90% and 38.10%, respectively. Both liposomal amphotericin B and posaconazole were given in all patients except one. CONCLUSION: A high suspicion of AIFRS should be kept in patients with recent COVID-19 infection who received steroids and presenting with headache, facial pain, and/or facial swelling. Asymptomatic COVID-19 carriers and COVID-19 vaccinated candidates are also observed to develop AIFRS, although the exact immuno-pathogenesis is still unknown. Prompt diagnosis and early management are vital for a favorable outcome.

Risk Indicators for Hearing Impairment in Neonates.

To identify relation between prevalence of hearing impairment and risk factors at the time of birth in high risk neonates. A year-long study is performed at HBT Medical College and Dr. R. N. Cooper Municipal General Hospital, Vile Parle (west), Mumbai. The Study comprised of 478 high risk babies born between March 2016 and February 2017 and admitted in NICU of the hospital. Out of these 68 babies were either transferred to higher centre or took discharge against medical advice. A total of 410 babies completed the study. Additional 21 eligible babies died in NICU during the study. A Prospective Observational Study is used to analyse the findings of the study. Skew analysis of spread of prevalence across risk factors is used to establish higher prevalence of hearing impairment in high risk neonates. Cramer's Moment Coefficient of Skewness and Corresponding Test Statistics are used for studying the skewness. Out of 410 babies screened during the study 5 babies were diagnosed with profound SNHL. The risk factors associated with babies is plotted against frequency of babies with Profound SNHL. The plot is found to be un-uniformly distributed and shows inclination towards higher prevalence with high risk babies. Sample skewness (G1) value - 0.81 indicates moderately negative skewed sample. This means the number of babies with profound SNHL found by 3 stage screening is moderately concentrated at high risk weightage.

Venolymphatic vascular malformation of the parotid gland extending into the parapharyngeal space: A rare presentation.

Vascular malformations (VMs) are structural malformations of vascular development causing soft tissue abnormality with functional and esthetic impairment. They are named by their predominant vessel type as arterial, venous, lymphatic or mixed types. VMs of the parotid gland are extremely rare and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management. Till 2013, only fifty cases of VMs of the parotid have been described in the literature. We present a case of a venolymphatic malformation of the parotid gland extending into the parapharyngeal space in a 21-year-old male who presented with a swelling on the left side of the face extending into the neck. Diagnosis was suggested by ultrasonography and computed tomography scan and was confirmed by magnetic resonance imaging examination. Complete surgical excision of the lesion was done with a favorable outcome. Diagnosis was confirmed based on histopathology and immunohistochemical studies.

Lymphangiomatous Polyp of Palatine Tonsil in A Child Presenting with Dysphagia and Dysarthria.

Pedunculated lymphangiomatous polyps of the tonsil are rare benign lesions that have been described in literature using varied nomenclature. Majority of the cases have been reported in adults with varying clinical symptoms. We report a case of lymphangiomatous polyp of left palatine tonsil in a 14-year-old male child who presented with dysphagia and dysarthria. Clinical examination revealed a large pedunculated polyp arising from upper pole of left tonsil. Patient underwent left tonsillectomy with excision of the polyp. Based on histopathological features a diagnosis of pedunculated lymphangiomatous polyp was made. We discuss the clinical and histopathological features of this lesion with differential diagnosis and short review of literature.